Histopathological Examination

The pouch wall consists of a normal number of ganglion cells, although a few authors have found reduced and very small ganglion cells [7,9,13,22,23]. Nerve bundle hypertrophy has also been reported, but is not the regular feature [22]. Congestion of the mucosa and focal hemorrhages are seen commonly [22,23]. In a detailed review of these cases, the authors found the following histological features in patients with CPC:

1. In most cases, the muscle coat did not have the normal differentiation of the inner circular and the outer longitudinal muscles. The muscles were also arranged in a decussating pattern. The circular muscle was incomplete in 50% of cases. The wall of the blood vessels was normal (Fig. 11.7 A).

2. The ganglion cells were mature and present in all cases, with the presence of normal or occasionally hypertrophic nerve bundles. However, giant ganglia were seen in 10% of cases (Fig. 11.7 B).

Fig. 11.2 Complete CPC with short length of normal bowel, cecum, and appendix. The remaining normal colon is not sufficient for pull-through procedure

Fig. 11.3 Incomplete CPC with dilated lower end of colon and Fig. 11.4 CPC showing abnormal blood vessels anorectal malformation. The descending colon is normal and can easily be used for performing a pull-through procedure

The most characteristic finding was disorganization of the muscle coat in an arborizing manner [22]. This is possibly responsible for the absence of normal peristaltic activity in these cases, requiring the removal of the dilated pouch and retaining only the normal bowel.

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